scholarly journals Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor

2014 ◽  
Vol 5 (2) ◽  
pp. 178-181 ◽  
Author(s):  
Eugene H. Chang ◽  
Xiao Xiao Tang ◽  
Viral S. Shah ◽  
Janice L. Launspach ◽  
Sarah E. Ernst ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Chronic Sinusitis ◽  
Medical Reversal

Management of Chronic Sinusitis in the Adult Cystic Fibrosis Patient

1998 ◽  
Vol 107 (11) ◽  
pp. 946-952 ◽  
Author(s):  
David J. Halvorson ◽  
John R. Dupree ◽  
Edward S. Porubsky
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Chronic Sinusitis

Rhinosinusitis in the Pediatric Patient with Cystic Fibrosis

2014 ◽  
Vol 10 (3) ◽  
pp. 198-201 ◽  
Author(s):  
Christopher Fundakowski ◽  
Rosemary Ojo ◽  
Ramzi Younis
Keyword(s):  
Cystic Fibrosis ◽  
Autosomal Recessive ◽  
Pediatric Patients ◽  
Surgical Intervention ◽  
Chronic Sinusitis ◽  
Genetic Disorder ◽  
Symptomatic Relief ◽  
Recurrence Rates ◽  
Polyp Recurrence ◽  
Sinonasal Polyposis

Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.

scholarly journals P258 CFTR modulator theratyping and functional impact of the rare CFTR genotype W57G/A234D in a cystic fibrosis patient

2019 ◽  
Vol 18 ◽  
pp. S130
Author(s):  
F. Pauro ◽  
F. Rescigno ◽  
A. Farinazzo ◽  
V. Esposito ◽  
K. Kleinfelder ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Functional Impact

Pulmonary infection secondary to Blastobotrys raffinosifermentans in a cystic fibrosis patient: Review of the literature

Mycoses ◽  
2021 ◽  
Author(s):  
Mohanad Al‐Obaidi ◽  
Hamid Badali ◽  
Connie Cañete‐Gibas ◽  
Hoja P. Patterson ◽  
Nathan P. Wiederhold
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Pulmonary Infection ◽  
Review Of The Literature

Paranasal Sinus Development in Chronic Sinusitis, Cystic Fibrosis, and Normal Comparison Population: A Computerized Tomography Correlation Study

1997 ◽  
Vol 11 (4) ◽  
pp. 275-282 ◽  
Author(s):  
Hung Jeff Kim ◽  
Ellen M. Friedman ◽  
Marcelle Sulek ◽  
Newton O. Duncan ◽  
Charles McCluggage
Keyword(s):  
Cystic Fibrosis ◽  
Maxillary Sinus ◽  
Paranasal Sinus ◽  
Chronic Sinusitis ◽  
Correlation Study ◽  
Uncinate Process ◽  
Ct Scans ◽  
Lateral Nasal Wall ◽  
Transverse Diameter ◽  
Sinus Disease

Chronic sinus disease in patients with and without cystic fibrosis may have an impact on the pattern of paranasal sinus pneumatization. Arrest of pneumatization has been reported in both of these conditions. To assess the development of the paranasal sinuses in relationship to chronic sinusitis and cystic fibrosis (CF), a retrospective review of coronal CT scans of the age-matched patients with no previous sinus disease, patients with chronic sinusitis, and cystic fibrosis patients was conducted. The patients’ ages ranged from 4 to 17 years. The maxillary sinus volume, anteroposterior diameter, and greatest transverse diameter and height were determined using image analysis software after the coronal CT scans were scanned into Macintosh computer. The size of the maxillary sinus increased with advancing age in the control and chronic sinusitis group, but not in the patients with cystic fibrosis. The patients with cystic fibrosis had a statistically significant smaller maxillary sinus size. Approximately 50% of the patients with chronic sinusitis had anatomic anomalies, the most common being paradoxical middle turbinates. The CT scans of CF patients were characterized by uncinate process demineralization and medial displacement of the lateral nasal wall in the middle meatus, and decreased maxillary sinus pneumatization.

A Novel Depsipeptide Produced by Paenibacillus alvei 32 Isolated from a Cystic fibrosis Patient

2012 ◽  
Vol 5 (1) ◽  
pp. 18-25 ◽  
Author(s):  
Romain Chevrot ◽  
Sandrine Didelot ◽  
Larissa Van den Bossche ◽  
Fatoumata Tambadou ◽  
Thibault Caradec ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Paenibacillus Alvei

Adult Cystic Fibrosis Presenting with Nasal Polyposis and Chronic Sinusitis

1994 ◽  
Vol 8 (5) ◽  
pp. 237-240 ◽  
Author(s):  
Erica R. Thaler ◽  
Sean M. Smullen ◽  
David W. Kennedy
Keyword(s):  
Cystic Fibrosis ◽  
Nasal Polyposis ◽  
Chronic Sinusitis ◽  
Sweat Testing ◽  
General Aspects ◽  
Recurrent Nasal Polyposis

The diagnosis of cystic fibrosis is important to the otolaryngologist because of the association with chronic sinusitis and nasal polyposis. Eighty-five percent of patients are diagnosed under the age of 15, and diagnosis beyond age 20 is uncommon. We present two patients over 35 years of age in whom the diagnosis of cystic fibrosis was made during the course of workup and treatment for recurrent nasal polyposis and chronic sinusitis. To our knowledge, only one prior such case has been reported. This paper will provide a brief overview of the general aspects of the disease, discuss otolaryngic manifestations and management, and recommend indications for sweat testing in the adult.

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